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Adamts13 ADAM metallopeptidase with thrombospondin type 1 motif 13 [ Mus musculus (house mouse) ]

Gene ID: 279028, updated on 25-Apr-2024

Summary

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Official Symbol
Adamts13provided by MGI
Official Full Name
ADAM metallopeptidase with thrombospondin type 1 motif 13provided by MGI
Primary source
MGI:MGI:2685556
See related
Ensembl:ENSMUSG00000014852 AllianceGenome:MGI:2685556
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
Gm710; vWF-CP; ADAM-TS13; ADAMTS-13
Summary
This gene encodes a member of "a disintegrin and metalloproteinase with thrombospondin motifs" (ADAMTS) family of multi-domain matrix-associated metalloendopeptidases that have diverse roles in tissue morphogenesis and pathophysiological remodeling, in inflammation and in vascular biology. In certain mouse strains (C57BL/6, for example) an intracisternal A-type particle (IAP) retrotransposon sequence is located in the intron 23 that causes an alternate splicing event resulting in a shorter transcript variants encoding shorter isoforms. The encoded preproprotein undergoes proteolytic processing to generate an active enzyme that cleaves von Willebrand factor (VWF) in circulating blood. [provided by RefSeq, Jul 2016]
Expression
Biased expression in liver adult (RPKM 2.2), liver E18 (RPKM 1.0) and 14 other tissues See more
Orthologs
human all
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Genomic context

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Location:
2 A3; 2 19.14 cM
Exon count:
31
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 2 NC_000068.8 (26863363..26899638)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 2 NC_000068.7 (26973361..27009625)

Chromosome 2 - NC_000068.8Genomic Context describing neighboring genes Neighboring gene surfeit gene 4 Neighboring gene STARR-positive B cell enhancer ABC_E2605 Neighboring gene serine/threonine kinase-like domain containing 1 Neighboring gene REX4, 3'-5' exonuclease Neighboring gene CapStarr-seq enhancer MGSCv37_chr2:26819876-26820059 Neighboring gene predicted gene, 36649 Neighboring gene STARR-positive B cell enhancer ABC_E7799 Neighboring gene calcium channel flower domain containing 1 Neighboring gene STARR-positive B cell enhancer ABC_E5901 Neighboring gene STARR-positive B cell enhancer ABC_E7800 Neighboring gene solute carrier family 2 (facilitated glucose transporter), member 6

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

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Related articles in PubMed

  1. Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function. Schelpe AS, et al. Blood Adv, 2020 Mar 24. PMID 32196558, Free PMC Article
  2. Formation and Resolution of Pial Microvascular Thrombosis in a Mouse Model of Thrombotic Thrombocytopenic Purpura. Adili R, et al. Arterioscler Thromb Vasc Biol, 2019 Sep. PMID 31340669, Free PMC Article
  3. ADAMTS13 Deficiency Shortens the Life Span of Mice With Experimental Diabetes. Cassis P, et al. Diabetes, 2018 Oct. PMID 29976618
  4. Long-Term Prevention of Congenital Thrombotic Thrombocytopenic Purpura in ADAMTS13 Knockout Mice by Sleeping Beauty Transposon-Mediated Gene Therapy. Verhenne S, et al. Arterioscler Thromb Vasc Biol, 2017 May. PMID 28254814
  5. N-acetylcysteine in preclinical mouse and baboon models of thrombotic thrombocytopenic purpura. Tersteeg C, et al. Blood, 2017 Feb 23. PMID 28011677

See all (87) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. ADAMTS13 deficiency exacerbates neuroinflammation by targeting matrix metalloproteinase-9 in ischemic brain injury.
    Title: ADAMTS13 deficiency exacerbates neuroinflammation by targeting matrix metalloproteinase-9 in ischemic brain injury.
  2. Regulation of von Willebrand factor by ADAMTS13 ameliorates lipopolysaccharide-induced lung injury in mice.
    Title: Regulation of von Willebrand factor by ADAMTS13 ameliorates lipopolysaccharide-induced lung injury in mice.
  3. ADAMTS-13-regulated nuclear factor E2-related factor 2 signaling inhibits ferroptosis to ameliorate cisplatin-induced acute kidney injuy.
    Title: ADAMTS-13-regulated nuclear factor E2-related factor 2 signaling inhibits ferroptosis to ameliorate cisplatin-induced acute kidney injuy.
  4. Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function.
    Title: Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function.
  5. Endothelial CD40 Mediates Microvascular von Willebrand Factor-Dependent Platelet Adhesion Inducing Inflammatory Venothrombosis in ADAMTS13 Knockout Mice.
    Title: Endothelial CD40 Mediates Microvascular von Willebrand Factor-Dependent Platelet Adhesion Inducing Inflammatory Venothrombosis in ADAMTS13 Knockout Mice.
  6. These results support a synergistic effect of severe ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy in mice.
    Title: Synergistic effects of ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy.
  7. ADAMTS13 knockout promotes the occurrence of early brain injury after experimental subarachnoid hemorrhage.
    Title: Role of von Willebrand factor and ADAMTS-13 in early brain injury after experimental subarachnoid hemorrhage.
  8. ADAMTS13-vWF axis is partially involved in the pathophysiology of kidney ischemic reperfusion injury.
    Title: ADAMTS13-von Willebrand factor axis is involved in the pathophysiology of kidney ischaemia-reperfusion injury.
  9. Adamts13 deficiency in obese mice promotes hepatic microthrombosis.
    Title: ADAMTS13 deficiency promotes microthrombosis in a murine model of diet-induced liver steatosis.
  10. results suggest that ADAMTS13 controls key steps of ischemic vascular remodeling and that recombinant ADAMTS13 is a putative therapeutic avenue for promoting stroke recovery.
    Title: ADAMTS13 controls vascular remodeling by modifying VWF reactivity during stroke recovery.
Submit: New GeneRIF Correction See all GeneRIFs (54)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)

General gene information

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Markers

Homology

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables endopeptidase activity IMP
Inferred from Mutant Phenotype
more info
 PubMed 
enables endopeptidase activity ISO
Inferred from Sequence Orthology
more info
  
enables hydrolase activity IEA
Inferred from Electronic Annotation
more info
  
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables metalloendopeptidase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables metalloendopeptidase activity ISO
Inferred from Sequence Orthology
more info
  
enables metallopeptidase activity IEA
Inferred from Electronic Annotation
more info
  
enables peptidase activity ISO
Inferred from Sequence Orthology
more info
  
Process Evidence Code Pubs
acts_upstream_of_or_within blood coagulation IEA
Inferred from Electronic Annotation
more info
  
involved_in extracellular matrix organization IBA
Inferred from Biological aspect of Ancestor
more info
  
acts_upstream_of_or_within hemostasis IEA
Inferred from Electronic Annotation
more info
  
involved_in peptide catabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in protein catabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in proteolysis IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in proteolysis ISO
Inferred from Sequence Orthology
more info
  
Component Evidence Code Pubs
is_active_in extracellular matrix IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in extracellular region IEA
Inferred from Electronic Annotation
more info
  
located_in extracellular space ISO
Inferred from Sequence Orthology
more info
  

General protein information

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Preferred Names
A disintegrin and metalloproteinase with thrombospondin motifs 13
Names
a disintegrin-like and metallopeptidase (reprolysin type) with thrombospondin type 1 motif, 13
a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13
vWF-CP mRNA for von Willebrand factor-cleaving
vWF-cleaving protease
von Willebrand factor-cleaving protease
NP_001001322.1
NP_001277392.1
NP_001277393.1
NP_001277394.1
XP_006498163.1
XP_006498166.1
XP_006498167.1

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001001322.2 → NP_001001322.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform 1 preproprotein

    See identical proteins and their annotated locations for NP_001001322.1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (1) represents the longest transcript and encodes the longest isoform (1).
    Source sequence(s)
    AB095445, AB112362
    Consensus CDS
    CCDS15820.1
    UniProtKB/Swiss-Prot
    A2ALB4, Q769J6, Q76LW1
    Related
    ENSMUSP00000099955.4, ENSMUST00000102891.4
    Conserved Domains (3) summary
    smart00209
    Location:392 → 444
    TSP1; Thrombospondin type 1 repeats
    cd04273
    Location:82 → 286
    ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
    pfam05986
    Location:565 → 683
    ADAM_spacer1; ADAM-TS Spacer 1

  2. NM_001290463.1 → NP_001277392.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform 2 precursor

    Status: REVIEWED

    Description
    Transcript Variant: This variant (2) uses an alternate in-frame splice site in the central coding region, compared to variant 1, resulting in an isoform (2) that is shorter than isoform 1. This isoform (2) may undergo proteolytic processing similar to isoform 1.
    Source sequence(s)
    AB095445, AB112362
    UniProtKB/Swiss-Prot
    Q769J6
    Conserved Domains (3) summary
    smart00209
    Location:392 → 444
    TSP1; Thrombospondin type 1 repeats
    cd04273
    Location:82 → 286
    ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
    pfam05986
    Location:565 → 683
    ADAM_spacer1; ADAM-TS Spacer 1

  3. NM_001290464.1 → NP_001277393.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform IAP-a precursor

    Status: REVIEWED

    Description
    Transcript Variant: This variant (IAP-a) lacks seven 3' exons but instead contains an alternate 3' terminal exon, and it thus differs in the 3' coding region and 3' UTR, compared to variant 1. The encoded isoform (IAP-a) has a distinct C-terminus and is shorter than isoform 1. This isoform (IAP-a) may undergo proteolytic processing similar to isoform 1.
    Source sequence(s)
    AL773563, AL845266
    Consensus CDS
    CCDS71010.1
    UniProtKB/TrEMBL
    A2ALB3, A7LM19
    Related
    ENSMUSP00000014996.8, ENSMUST00000014996.14
    Conserved Domains (3) summary
    smart00209
    Location:392 → 444
    TSP1; Thrombospondin type 1 repeats
    cd04273
    Location:82 → 286
    ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
    pfam05986
    Location:565 → 683
    ADAM_spacer1; ADAM-TS Spacer 1

  4. NM_001290465.1 → NP_001277394.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform IAP-b precursor

    Status: REVIEWED

    Description
    Transcript Variant: This variant (IAP-b) uses an alternate in-frame splice site and lacks an alternate in-frame exon in the central coding region, and it also lacks seven 3' exons but instead contains an alternate 3' terminal exon and thus differs in the 3' coding region and 3' UTR, compared to variant 1. The encoded isoform (IAP-b) has a distinct C-terminus and is shorter than isoform 1. This isoform (IAP-b) may undergo proteolytic processing similar to isoform 1.
    Source sequence(s)
    AL773563, AL845266, EU034706
    UniProtKB/TrEMBL
    A7LM19
    Conserved Domains (2) summary
    smart00209
    Location:392 → 444
    TSP1; Thrombospondin type 1 repeats
    cd04273
    Location:82 → 286
    ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000068.8 Reference GRCm39 C57BL/6J

    Range
    26863363..26899638
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_006498100.5 → XP_006498163.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform X1

    Conserved Domains (3) summary
    smart00209
    Location:392 → 444
    TSP1; Thrombospondin type 1 repeats
    cd04273
    Location:82 → 286
    ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
    pfam17771
    Location:306 → 376
    ADAM_CR_2; ADAM cysteine-rich domain

  2. XM_006498104.5 → XP_006498167.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform X3

    UniProtKB/TrEMBL
    A7LM19
    Conserved Domains (4) summary
    smart00209
    Location:392 → 444
    TSP1; Thrombospondin type 1 repeats
    cd04273
    Location:82 → 286
    ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
    pfam05986
    Location:565 → 683
    ADAM_spacer1; ADAM-TS Spacer 1
    pfam17771
    Location:306 → 376
    ADAM_CR_2; ADAM cysteine-rich domain

  3. XM_006498103.5 → XP_006498166.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform X2

    UniProtKB/TrEMBL
    A7LM19
    Conserved Domains (4) summary
    smart00209
    Location:392 → 444
    TSP1; Thrombospondin type 1 repeats
    cd04273
    Location:82 → 286
    ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
    pfam05986
    Location:565 → 683
    ADAM_spacer1; ADAM-TS Spacer 1
    pfam17771
    Location:306 → 376
    ADAM_CR_2; ADAM cysteine-rich domain

RNA

  1. XR_001782991.3 RNA Sequence

Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q769J6.1 GenPept UniProtKB/Swiss-Prot:Q769J6

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